Intraocular Medulloepitheliomae (Clinical Case Series)

• Published in: Oftalmologii͡a Vol. 17; no. 3s; pp. 653 - 663
• Main Authors: Saakyan, S. V., Tsygankov, A. I., Maybogin, A. M., Amiryan, A. G., Khlgatyan, M. R., Zhiltsova, M. G., Khoroshilova‑Maslova, I. P., Zakharova, G. P.
• Format: Journal Article
• Language: English; Russian
• Published: Ophthalmology Publishing Group 13.11.2020
• Subjects: внутриглазная опухоль; медуллобластома; медуллоэпителиома; ретинобластома; увеальная меланома
• ISSN: 1816-5095; 2500-0845
• DOI: 10.18008/1816-5095-2020-3S-653-663

Purpose. Retrospective analysis of clinical, instrumental, cytological and pathological features of intraocular medulloepithelioma based on our own experience in the period from 2005 to 2018. Patients and Methods. For a specified period of time in the department of ocular oncology and radiology in Moscow Helmholtz Research Institute of Eye Diseases we examined 9 patients aged from 1 to 44 years (5 men and 4 women), with a confirmed histological diagnosis of intraocular medulloepithelioma (medulloblastoma).  The average age of patients in assessing vital status was 15.8 ± 13.5 years, the average age at the time of  making the diagnosis “intraocular neoplasm” was 9.5 ± 11 years. All patients underwent a comprehensive clinical and instrumental ophthalmologic examination, which included visometry, tonometry, perimetry, biomicroscopy, ophthalmoscopy, Doppler ultrasound (ACUSON, USA), as well as general physical examination. Analysis of the initial status (before hospitalization) was carried out retrospectively according to the data of outpatient records. In one case, iridocyclosclerectomy was performed, in eight cases — enucleation of the affected eye followed by histopathological examination, as well as analysis of long-term results of treatment. Results. A detailed analysis of nine clinical cases is presented, including typical clinical features, echographic signs of a meduloepithelioma (heterogeneous structure of the tumor, anechoic inclusions (cysts) in the  thickness of the tumor) are presented. According to the localization, we observed the tumor of the ciliary body (n = 3), the ciliary body and iris (n = 2), the retina (n = 1), the retina, the choroid and the optic nerve disc (n = 1), the ciliary body and the choroid (n = 1) and the choroid with invasion in extraocular muscles and optic nerve (n = 1). In 8 cases, enucleation was performed, one case was preceded by an iridectomy and a fine- needle aspiration biopsy. One patient underwent local conservative treatment (iridocyclosclerectomy) in the  presence of a tumor of the ciliary body and iris. Pathological and cytological features of the tumor were presented. 8 of 9 patients were alive and had no signs of metastases. One patient died 3 years after treatment.  Conclusions. Despite the relative rarity, medulloepithelioma should be included in the differential-diagnostic series in patients with suspected retinoblastoma, non-pigmented uveal melanoma, adenocarcinoma of the  retinal pigment epithelium, and other rare intraocular neoplasms.